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Sickle cell disease (SCD)

Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease.

Children with Sickle Cell Disease (SCD) are at risk for developing multiple intracranial aneurysms, and a high index of suspicion must be maintained during the interpretation of routine magnetic resonance imaging or angiography of the brain 1).

Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease

Case reports


A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. During his hospital stay he developed rapid deterioration of his mental status and computed topography revealed a spontaneous massive epidural hematoma with mass effect and midline shift with Kernohan notch phenomenon for which urgent craniotomy and evacuation was done.This is the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan's notch phenomena within few hours with rapid clinical deterioration. The etiopathogenesis and the rare presentation are discussed in detail in this case report 2)

Lo Presti et al. present the case of a 15-year-old boy with Sickle cell disease SCD-associated moyamoya disease harboring a intracranial pial arteriovenous fistula pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence 3).

Saini S, Speller-Brown B, Wyse E, Meier ER, Carpenter J, Fasano RM, Pearl MS. Unruptured Intracranial Aneurysms in Children With Sickle Cell Disease: Analysis of 18 Aneurysms in 5 Patients. Neurosurgery. 2015 Feb 12. [Epub ahead of print] PubMed PMID: 25710108.
Yogarajah M, Agu CC, Sivasambu B, Mittler MA. HbSC Disease and Spontaneous Epidural Hematoma with Kernohan's Notch Phenomena. Case Rep Hematol. 2015;2015:470873. Epub 2015 Oct 21. PubMed PMID: 26576305.
Lo Presti A, Weil AG, Fallah A, Peterson EC, Niazi TN, Bhatia S. Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report. J Neurosurg Pediatr. 2015 May 22:1-5. [Epub ahead of print] PubMed PMID: 26053963.
sickle_cell_disease.txt · Last modified: 2015/11/18 22:18 (external edit)